Erythroderma: a prospective study of 309 patients followed for 12 years in a tertiary center.

Erythroderma is characterized by erythema and scaling affecting more than 80% of the body surface area. It is potentially life-threatening, and diagnosis of the underlying disease is a challenge. Despite laboratory improvements, many cases remain idiopathic. We aimed to analyze clinical and laboratory findings of 309 erythrodermic patients to find clues to the etiologic diagnosis. We performed a prospective study at the University of São Paulo Medical School, from 2007 to 2018, with patients with acquired erythroderma. Clinical, laboratory, histology, and molecular biology data were collected. The median age at diagnosis was 57 years, with a male-to-female ratio of 2.2. Eczema was the most frequent etiology (20.7%), followed by psoriasis (16.8%), Sézary syndrome (12.3%), drug eruption (12.3%), atopic dermatitis (8.7%), and mycosis fungoides (5.5%). Other diagnoses (6.8%) included pemphigus foliaceous, paraneoplastic erythroderma, adult T-cell leukemia/lymphoma, dermatomyositis, pityriasis rubra pilaris, lichen planus, bullous pemphigoid, and leprosy. In 52 patients (16.8%), it was not possible to elucidate erythroderma etiology. Atopic dermatitis developed erythroderma at an earlier age (median 25 years; P = 0.0001). Acute onset was associated with drug reactions and atopic dermatitis (median time from erythroderma to diagnosis of 1 and 1.5 months, respectively; P = 0.0001). Higher immunoglobulin E levels were observed in atopic dermatitis (median 24,600 U/L; P = 0.0001). Histopathology was helpful and was consistent with the final diagnosis in 72.4%. Monoclonal T-cell proliferation in the skin was observed in mycosis fungoides (33.3%) and Sézary syndrome (90.9%). At the last assessment, 211 patients (69.3%) were alive with disease, 65 (21.7%) were alive without disease, and 27 (9.1%) died with active disease. Erythroderma is a challenging syndrome with a difficult diagnostic approach. Younger age and higher immunoglobulin E levels are associated with atopic dermatitis; acute onset is observed in drug eruptions and atopic dermatitis. Histopathology and molecular biology tests are essential tools in the investigation of erythroderma.

[Role of intercellular slit-like contacts (connexins) in the pathogenesis of erythroderma]. / Rol' mezhkletochnykh shchelevidnykh kontaktov (konneksinov) v patogeneze éritrodermii.

Erythroderma is a skin lesion characterized by redness, swelling, infiltration, and desquamation of greater than 90% of the skin. The etiology of erythroderma is not completely clear and the lesion can be manifestations of various chronic dermatoses, including atopic dermatitis, psoriasis, eczema, and toxicodermia, and be represented by erythrodermic mycosis fungoides. The pathogenesis of erythroderma especially at the genetic level remains little studied. Thus, one disease (erythroderma) can be a manifestation of different dermatoses and have similar clinical and histological signs. This paper gives a review of modern literature on the study of erythroderma in terms of morphology and genetic aspects.

Proposed ICDRG Classification of the Clinical Presentation of Contact Allergy.

The International Contact Dermatitis Research Group proposes a classification for the clinical presentation of contact allergy. The classification is based primarily on the mode of clinical presentation. The categories are direct exposure/contact dermatitis, mimicking or exacerbation of preexisting eczema, multifactorial dermatitis including allergic contact dermatitis, by proxy, mimicking angioedema, airborne contact dermatitis, photo-induced contact dermatitis, systemic contact dermatitis, noneczematous contact dermatitis, contact urticaria, protein contact dermatitis, respiratory/mucosal symptoms, oral contact dermatitis, erythroderma/exfoliative dermatitis, minor forms of presentation, and extracutaneous manifestations.

Histopathologic diagnosis of lymphomatous versus inflammatory erythroderma: a morphologic and phenotypic study on 47 skin biopsies.

Erythroderma may be secondary to a cutaneous T-cell lymphoma (CTCL) and various other erythrodermic inflammatory dermatoses (EID), and their histopathologic distinction is often difficult. The aim of this study was to determine if morphological parameters, namely: the presence of b-catenin, and JunB (previously shown to be expressed by CTCL cells), the epidermal CD8:CD3 ratio, and CD30 expression may help in the histopathologic diagnosis of erythroderma, especially in differentiating CTCL and EID. We retrospectively reviewed a series of 47 skin biopsies from patients with erythroderma (18 CTCL and 29 EID). The diagnosis of each case was established using clinical, biological and histopathologic data. After a blind assessment of the hematoxylin--eosin--safran stained slides, a correct diagnosis of the underlying cause of erythroderma was made only in 31% of cases. A correct differential diagnosis between lymphoma and EID was done with certainty in 57% of cases. Various morphologic and phenotypic parameters were then recorded and we compared their frequency in the CTCL versus the EID group. With the exception of atypical lymphocytes, the moderate to high density of dermal infiltrates and Pautrier microabcesses, only found in CTCL, no morphologic parameter was found to be specific of CTCL, although single lymphocytes epidermotropism, telangiectasias, and slight lymphocytic dermal infiltrate were significantly more frequent in EID. A low (<10%) CD8:CD3 ratio in the epidermal lymphocytic infiltrate and dermal CD30+ lymphocytes were significantly more frequent in CTCL. JunB expression by lymphocytes was specific of CTCL, but was inconstant in our series (17%). We found ß-catenin expression in a minority of cases from both the CTCL and EID groups. Among EID, dermal suprapapillary thinning was specific of psoriasis. Neutrophils exocytosis and edema of papillary dermis were significantly more frequent in psoriasis, and spongiosis was more frequent in eczema. In conclusion, few morphological and phenotypical parameters are helpful in making a differential diagnosis between erythrodermic CTCL and EID using paraffin embedded skin biopsies.

A modified staging classification for cutaneous T-cell lymphoma.

BACKGROUND: Despite refinements in the diagnosis of cutaneous T-cell lymphoma (CTCL), since 1979 there have been no changes to the staging of CTCL used to classify mycosis fungoides and Sézary syndrome. OBJECTIVE: We reviewed the current staging of CTCL and examined the usefulness of a new staging scheme for mycosis fungoides and Sézary syndrome. METHODS: We determined overall survival of 450 patients with mycosis fungoides and Sézary syndrome using the current and modified staging classifications. RESULTS: There were no significant differences between survival of patients with stage IB (patches/plaques involving greater than 10% body surface area) and IIA (peripheral adenopathy) disease and of patients with stage IIB (tumor) and III (erythroderma) disease. There was a significant difference in survival between patients with extensive patch versus extensive plaque stage disease. Modification of the current classification by splitting T2 into patch versus plaque stage disease and incorporating tumors and erythroderma into stage III proved superior to the current scheme in predicting overall survival. CONCLUSION: Modification of the current staging classification for CTCL yields subgroups useful in the prognostic assessment of CTCL.

Erythroderma: analysis of 247 cases

Foi analisado o perfil de 247 doentes com eritrodermia em um período de 23 anos, de janeiro de 1962 a março de 1985, com o período de seguimento variando de 1 a 26 anos. Os doentes se apresentavam com eritema universal, descamaçäo e prurido com mais de 2 meses de duraçäo e a idade variava de 16 a 60 anos. A psoríase foi a doença associada mais freqüente, com uma proporçäo estimada de 44,9 por cento, as reaçöes cutâneas ao uso de drogas contribuíram com 7,3 por cento do total de casos e a associaçäo com reticuloses mostrou uma proporçäo de 4,1 por cento. A eritrodermia permaneceu como de causa desconhecida em 29,2 por cento dos casos. Näo foram observadas diferenças entre os sexos no que diz respeito à doença associada. Um ou mais resultados anátomo patológicos das biópsias de pele, em conjunto com o quadro clínico, foi diagnóstico ou sugestivo do diagnóstico da doença associada em 63,6 por cento dos casos. Recomendam-se biópsias de pele seriadas como o melhor método para a elucidaçäo diagnóstica da eritrodermia. Ao nível de significância P=0,05, foi encontrada uma proporçäo homem/mulher em 2 : 1. Especula-se se o agente causal da eritrodermia estaria relacionado à exposiçäo diferenciada entre os sexos a antígenos do meio ambiente

Lymph node classification systems in cutaneous T-cell lymphoma. Evidence for the utility of the Working Formulation of Non-Hodgkin's Lymphomas for Clinical Usage.

BACKGROUND: This study was undertaken to compare three classification schemes used to evaluate lymph nodes (LN) obtained from patients with cutaneous T-cell lymphoma (CTCL): a modified Rappaport classification, the National Cancer Institute-Veterans Administration (NCI-VA) classification based on the relative numbers of cerebriform cells in the paracortical areas, and the Dutch classification based on the presence of cerebriform cells with large nuclei in mycosis fungoides (MF) and diffuse infiltration by cerebriform cells in Sézary syndrome. METHODS: A study set of 195 LN obtained from patients with CTCL (MF, Sézary syndrome, and nonepidermotropic T-cell lymphomas) and 14 LN from patients with benign dermatoses was reviewed independently by three groups of pathologists familiar with each classification system. RESULTS: Each classification system provided useful prognostic information. However, contrary to prior reports, no significant difference in survival was apparent in patients with uneffaced LN when classified according to the NCI-VA (LN0-2 versus LN3) or Dutch (Gr0-1 versus Gr2) ratings. In addition, all classification systems demonstrated a poor survival time associated with effaced LN. By combining results from the modified Rappaport and Dutch classifications, three prognostic groups could be identified based on cell morphology: a low-grade category with a small cell histologic subtype (median survival time, 40 months); a high-grade immunoblastic subtype (median survival time, 9 months) composed of cells with an oval nucleus containing a large, usually solitary central nucleolus; and an intermediate-grade category composed of all cases without the distinctive small cell and immunoblastic morphologies (median survival time, 26 months). CONCLUSIONS: The authors propose that clearly involved LN in CTCL can be categorized on the basis of cell morphology into prognostic groups analogous to what has been proposed for the Working Formulation for Non-Hodgkin's Lymphomas for Clinical Usage.

Eritrodermia: análise de 247 casos / Erythroderma: analysis of 247 cases

Segundo a metodologia de estudo de casos, foram revisados os prontuários de 247 doentes com eritrodermia atendidos na Clínica de Dermatologia do Instituto Central do Hospital das Clínicas da Faculdade de Medicina da Universidade de Sao Paulo, de janeiro de 1962 a março de 1985, com períodos de seguimento de 1 a 26 anos. Foram considerados para o trabalho os doentes que apresentavam eritema e descamaçao universais acompanhados de prurigo, com duraçao acima de 2 meses. Nao foram incluídos os doentes com pênfigo foliáceo. Ao nível de significancia de 0,05 encontrou-se proporçao de homens com eritrodermia maior que a de mulheres (aproximadamente 2:1) e uma concentraçao maior de doentes eritrodérmicos na faixa de 16 a 60 anos de idade. No mesmo nível de significância nao se observou diferença entre os sexos na frequência das doenças associadas. Em 63,58 por cento das vezes, o exame histopatológico da pele, à luz da suspeita clínica e repetido, mostrou-se conclusivo ou sugestivo da doença associada à eritrodermia. A psoríase foi a doença associada mais frequente, com a verdadeira proporçao entre 37,8 por cento e 50,4 por cento de confiança causa da eritrodermia ficou indeterminada na proporçao verdadeira entre 22,6 por cento e 34,0 por cento, com 95 por cento de confiança

Eritrodermia em doente de lepra poupando as lesões maculosas (Casos clinicos)

Em caso de lepra maculosa com lesões eritemato-discromicas, uma eritrodermia por contato com a "Aroeira" respeitou tais lesões, que passaram a se distinguir agora por contraste "negativo".